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Large congenital melanocytic nevus

Disease definition

A rare skin hamartoma characterized by at least one pigmented skin lesion present at birth of more than 20 cm (large congenital melanocytic nevus; LCMN) or 40 cm (giant; GCMN) projected adult diameter. The primary lesion is composed of mutated melanocytes and often locally disorganized epidermal annexes or dermis, and presents with an elevated risk of malignant transformation to melanoma or, more rarely, other neoplasms in skin or central nervous system.

ORPHA:626

Classification level: Disorder
  • Synonym(s):
    • Congenital pigmented nevus
    • GMN
    • Giant congenital melanocytic nevus
    • Giant pigmented hairy nevus
    • LCMN
  • Prevalence: 1-9 / 100 000
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: Infancy, Neonatal
  • ICD-10: D22.9
  • ICD-11: 2F20.20
  • OMIM: 137550
  • UMLS: C1842036
  • MeSH: -
  • GARD: 2469
  • MedDRA: 10072036

Detailed information

Guidelines

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)
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